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Objective@#To assess the safety and effectiveness of sufficient, short-term platelet (PLT) transfusion for the surgery preparation of the infantile patients with Kasabach-Merritt phenomenon, who were insensitive to glucocorticoids.@*Methods@#The infantile cases were retrospectively analyzed during May 2011 to December 2016, who were clinically diagnosed as KMP and insensitive to glucocorticoids, received PLT transfusion and surgical resection. PLT transfusion in patients whose PLTC was less than 30×109/L, was 0.3 therapeutic dose(TD)/kg, and 0.2 TD/kg in PLTC≥30×109/L group. The maximum was 1 TD.Criteria of the PLT transrusion: 1 hour after the transfusion, the PLT count (PLTC) were tested and the corrected count increment of platelet (CCI) and practical platelet recovery (PPR) was calculated. PLTC ≥100×109/L, CCI>7.5×109/L and PPR>30% were defined as effective; while PLTC=(50-99)×109/L, CCI>7.5×109/L and PPR>30% as partial effective; PLTC<50×109/L, or CCI≤7.5×109/L, or PPR ≤30% were defined as ineffective. By reviewing the method and response of their PLT infusions, to figure out the most effective way in rising PLT, as a part of pre-operation treatment.@*Results@#There were 46 cases in the research. Based on the PLTC, CCI and PPR 1 hour after PLT transfusion, there were 44 effective transfusion, 2 patients with partial effectiveness, and no ineffective case. There was no allergic or heart failure happened in any cases. No critical potential complications of PLT transfusion occurred, including fluid and iron overload, alloimmunization to human leukocyte antigen and/or PLT antigen.@*Conclusions@#Pre-operative sufficient and short-term PLT infusions are more effective than low dose and long-term ones. They can create a more optimistic opportunity for surgical resections.
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Objective@#To define a new subtype of vascular anomaly, named fibro-adipose vascular anomaly(FAVA)and to discuss the methods of diagnosis and treatment in order to decrease the misdiagnosis rate and improve the recovery rate.@*Methods@#From Jan. 2011 to Dec. 2015, 34 cases with FAVA on the lower extremities received surgical treatment in our center. The clinical data were collected to summarize the imaging and pathological characteristics for diagnosis.@*Results@#The misdiagnosis rate was 76.5%(26/34) in all these 34 patients. The cure rate was 100% after operation. The patients were followed-up for 1-3 years(average, 19 months) with normal function and no recurrence.@*Conclusions@#FAVA is usually misdiagnosed as hemangioma or vascular malformation. The disease should be well defined to help the diagnosis and treatment. Surgical excision is one of the optional treatments.
ABSTRACT
Objective To analyze the surgical treatment of hemangioma of limbs complicated by Kasabach-Mcrritt phenomenon in children.Methods A retrospective study was carried out on 31 children with hemangioma of limbs complicated by Kasabach-Merritt phenomenon.Lower limbs were affected in 19 patients,and upper limbs in 12 patients.Twenty-six patients showed decreased platelet count ((2.6-60) × 109/L) and fibrinogen level (＜ 1 g/L),and 21 prolonged partial prothrombin time (＞ 50 seconds).All the patients who had responded poorly to medications including glucocorticoids received surgical treatment.Results Of these patients,30 were cured,and one died of massive blood loss.After surgical treatment,the platelet count returned to normal in 1-3 days in 25 patients,3-7 days in 4 patients,and more than 10 days in one patient.Conclusions For pediatric hemangiomas of limbs complicated by Kasabach-Merritt phenomenon,surgical treatment should be given as early as possible so as to reduce mortality in patients.